Tuesday, November 30, 2010

It wasn't bound to happen, but it's not surprising that it has. And don't worry. We've got your back. Also your eyes, mouth, and front. That's the official assessment from the good Dr. Soiffer, per Susan's visit to DFCI yesterday. In short, she has developed a mild to moderate case of GVH (graft vs. host), which has manifested as a body rash, mouth sores, and inflamed and glassy eyes . In case you don't remember what GVHD is, you can scroll down to the bottom of the blog where you'll find a description of the disease, the transplant procedure, and the risk factors in doing the transplant.

The condition isn't fun, but it isn't debilitating. Maria and I visited with Susan and Bob this past Saturday in Spencertown, and she was up to hosting a small family gathering (Leslie, Rudy, and John Henri Richardson, Nate and Callie, Charley Siegel, and us). With her body covered, as it was, you wouldn't have known.

That said, the condition has to be treated and watched. So, here's the summary from the DFCI visit: blood work results were basically good, except for a slight elevation in the liver numbers. Dr. Soiffer had Susan see an oral oncologist on staff, who prescribed a lip cream and is deciding between one of two different mouth rinses that are currently being evaluated in a clinical study. Susan will need to be enrolled in the study in order to receive the treatment, and that registration (which may require another trip to Boston next week) is currently in process. The good news about this study is that it does not compare the treatment to a placebo but rather pits the two medications against each other, head to head. So, Susan will be getting something, whatever it is, that is expected to work.

For her eyes, Susan still needs to see an oncological opthamologist (which will happen at DFCI if she returns next week, otherwise in NYC), and for her body rash, her own dermatologist (who did an oncology rotation at DFCI). So, there are still open issues, but a plan is in place to address them. In the meantime, please realize that while her condition needs to be treated and followed, it is not considered life threatening. She is uncomfortable but not miserable. It is not the "yechy-ness" of old.

Susan will need to return to DFCI in about a month as part of the study to assess the efficacy of whichever mouth rinse is prescribed for her. Hopefully by then the rash, and the GVH, will have subsided, if not disappeared entirely. You can read about it here when it happens.

Tuesday, October 5, 2010

Susan, accompanied by her ever faithful chauffeur and spouse, Bob, was in Boston yesterday to see Dr. Rob Soiffer, her ever faithful oncological transplant specialist. The tests run indicated no issues worth mentioning, Susan's outward appearance and her self-assessed condition were good, and Dr. S. pronounced himself satisfied. He swapped out one medication for a milder variant and indicated that he planned, over time, to wean Susan off most of the drugs she currently takes. A good marker for her progress is that Dr. Soiffer scheduled her next appointment for two months out. Remember back when it was every week?

As for this blog, is anyone still out there? At this point, the blog's audience, if any remnant of it remains, realizes that Susan has been doing well these last several months. Accordingly, blog entries are becoming briefer and more widely spaced. Soon they may become so brief and so widely spaced in time that they will, for all practical purposes, cease to exist, and that will be a good thing. Nevertheless, Susan's ever faithful blogster will remain at his post blogging until there is absolutely nothing left to blog because Susan has been pronounced officially "cured." And if these posts have already become, or will so become soon, something akin to the problematic sound or lack thereof of the proverbial tree falling in the forest with no one to hear (or, in this case, see), so be it. . . .

. . . all of which is to say, in typically long-winded fashion: "back in two months with the next update, whether you tune in to read it or not."

Tuesday, July 27, 2010

Short and Sweet: Susan had a good visit with Dr. Soiffer yesterday. There are a few test results that were not available on a same day basis, but what lab work did come back was decent. From a quantitative perspective, Susan is doing well at this point, and those of you who have seen or talked to her recently know that in general she's been feeling pretty good.

As you may remember, the further out from the transplant Susan gets, the less likely it is that she will have complications or setbacks. So what's happening (or not happening) now is goodness. However, while increasingly less likely, problems can still arise, so the appropriate position is "guardedly optimistic."

Wednesday, June 2, 2010

Hard to believe, but it was a year ago today that Susan received her transplant. For those fond of strolling down Memory Lane, I have dug into the archives to append the blog from that day (see below). As for today, here's how things stand:

Susan was to have gone to Boston yesterday for a liver biopsy, among other things, but she "called in sick" with a sore throat, so those procedures were postponed. By last night she was feeling better and today Susan and Bob drove up for her scheduled appointment with Dr. Soiffer. Her consultation came after the usual round of vital-signs-taking and blood tests, all the results of which are not yet available, but based upon what Soiffer did see, he pronounced her "doing very well at this point," especially given her rocky history over the past year.

To be more specific: those test results that were available by this afternoon were all good, including those that indicate how the liver is doing (e.g., bilirubin). The liver, as a potential locus for GVHD, has been, you'll remember, an ongoing concern, with a secondary manifestation of severe ascites. One of the rationales for the liver biopsy scheduled for yesterday was continuing ascites. I'm delighted to report that the ascites is now so diminished that Soiffer doesn't see a need for the liver biopsy.

The most interesting aspect of the day was the spate of re-vaccinations which Susan needed to have administered. A year ago, when she lost her marrow, she lost her vaccine-provided immunities. Today, like an infant, she was at the point where she was ready for her standard set of childhood vaccinations. Happy Anniversary and Happy Birthday, Susan!

As those of you who have had recent contact with Susan outside of this blog will know, after what seemed an interminable period of halting and uncertain progress, with two fair-to-good days followed by a really Y**** day, Susan has strung together an impressive number of good days. While she appears painfully thin, or fashionably slender, she has actually gained back 4 pounds recently and is now eating with a pretty good appetite. Woohoo! It's a far cry from cheering when she managed to down half of a grilled cheese sandwich.

The one year mark is often thought of as a watershed (informally, of course, as there's no clinical, or magical, boundary that separates day 364 from 366). But transplant patients who make it this far are seen as moving into clearer, less dangerous waters. No guarantees (when ever are there) but the progress is indisputable.

We'll wait, of course, for the remaining test results, but absent surprises, Susan's free to cruise along unmonitored until her next DFCI visit on July 9th. Until then . . .

The deed is done. The infusion began at approximately 10:15 today and was finished about an hour and a half later - turned out to be briefer than expected. And yes, for the major inflection point in Susan's disease, it was an underwhelming event. Nurse Karen simply hooked up the bag with the stem cells (the red bag in the picture above) and that was it. No drumrolls, no fireworks display, just a steady drip like any other coming from a bag through an IV line. If Susan did not know it was happening, she wouldn't have been able to tell.

Susan's team of 5 doctors came in at one point during the procedure, nodded sagely, clucked a bit, squabbled mildly and made up (just to show they were on the job), and then left without doing anything or offering any useful information or advice. Dr. Soifer (Susan's lead transplant specialist) stopped in a bit later to say hello and said everything was looking good.

Susan has no appetite but Nurse Karen says that's par for the course. I'll continue to nag her about her applesauce and fluids. Otherwise, she definitely looks better than she did yesterday and feels somewhat better - if she were eating and drinking, she'd feel better still, but that's a fine balancing act between nutrition and nausea.

Susan's room is a little like Grand Central - people are constantly popping in and out. In just the past hour we've had Nurse Karen bearing ice chips, Carol the Patient Care Coordinator with documents to sign, and Tammy the Social Worker for no discernible reason, but she was very sweet and she did leave her card.

And finally, Susan says "Hi. I'm glad it's over, but the real part is just starting." Couldn't coax more of a statement from her for the record at this point, but she says she may have more to say later, so stay tuned.

Wednesday, April 14, 2010

Susan and Bob went up to Dana Farber Monday, where they expected to meet with the gastroenterologist to get to the bottom of the ascites/liver issue. Susan was looking and feeling a bit peaked, though, and the blood work (which is always done first upon arrival) revealed a significantly depressed hematocrit, a sign of internal bleeding. Dealing with this put all other plans on hold.

An endoscopy was performed and it revealed bleeding ulcers, both esophogeal and gastric. Susan was hospitalized, put on Nexium intravenously, and given 4 units of blood. Today she was feeling much better -- nothing like a jolt of fresh blood to perk a body up -- and actually ate a good breakfast.

This afternoon they performed a needle biopsy to get a sample of the ascites fluid. The doctors are going to consider the results of the various samples they have taken and bring that information into an examination and consult on April 26th with the gastroenterologist being tasked to get to the bottom of Susan's condition. Paracentesis to drain the ascites fluid is also a likely treatment item when she returns on the 26th. As I write, she should be in the process of being released to return home to NYC, but with a number of adjusted and new medications.

Tune back in late on April 26th, when I expect to be blogging the results of Susan's next DFCI visit.

Tuesday, April 6, 2010

The good news is that there was no bad news resulting from Susan's visit to Dr. Soiffer on Monday. However, she continues to be something of a medical mystery. Ascites, the buildup and retention of fluid in the abdomen, is usually a byproduct of some kind of liver issue. Susan's ascites continues and seems to be associated with her maddening pattern of two or three good days followed by a bad day (remember "yech"?). So, what's the liver issue? What's going on gastrointestinally speaking? What's root cause? Nobody knows.

As Soiffer explained it, all the various tests she's had over the past several months -- endoscopy, MRI, liver ultrasound, et al. and ad infinitum -- seem to point in different, mutually exclusive directions. It's a puzzlement. He has enlisted his most preferred gastroenterologist, someone who was desired but not available in an earlier go-round on this matter, and this guy, we hope, will be able to figure it out. He has ordered an echocardiogram, to be done this week in NYC, and then a return visit to DFCI next Monday when Susan will undergo another paracentesis to remove excess fluid that's accumulated due to the ascites. Based on outcomes, combined with all previous data points, he will, we hope, have a better understanding of Susan's condition than has been hitherto achieved and will prescribe the appropriate course of action.

Meanwhile, let's keep this all in perspective. It's now more than 300 days since the procedure. The further out she gets, the better the prognosis for a lasting cure. While there's been some GVHD, which is the greatest threat to a transplant patient, it has not been particularly virulent. Over time, if the conflict hasn't escalated, the foreign and native armies just get worn out from fighting and decide to get along, intermingle, intermarry, until, ultimately, they can't tell themselves apart from each other.

Susan is experiencing more good days, days where she feels almost like a civilian, than bad. She's getting things done, getting out of the house (except on bad days). Let's see what this new doctor comes up with. I'll report back, via this blog, after her visit to him next week.

Tuesday, March 9, 2010

Top Line: Susan's not doing too badly, but the docs aren't sure what's going on

Yesterday Susan was up in Boston for an endoscopy and a consult with Dr. Soiffer, the oncologist who's overseeing her transplant and recovery.

The endoscopy revealed no signs of GVHD (graft vs. host disease) in her stomach or above; however, it did show something going on which they are uncertain about at this point.

The liver biopsy which Susan had a bit ago indicates GVHD affecting the liver, but it isn't a severe, debilitating case. The treatment for this, upon which she is about to embark, is low dosage steriods.

Susan continues with her pattern of a couple of good days followed by a bad day. Why she should experience this fairly regular cycle no one knows. Perhaps it's the GVHD, in which case the steroids may resolve the matter, or perhaps it's due to whatever it is that was noticed in the endoscopy, or perhaps it's something else entirely. Let's hope some clarity emerges at her next Soiffer visit, which will be in about a month.

Thursday, February 4, 2010

Susan, ferried by the indefatigable Bob, spent a couple of days in Boston this week. On Monday she had her usual round of blood work and saw Dr. Soiffer. For the most part, her numbers were good, although a couple of areas were suspect and bear watching. No need for details unless and until there's something specifically and clearly amiss.

Tuesday was the liver biopsy, accomplished under local anesthetic (and a tranquilizer). The process was an all day affair, start to finish, given necessary post-procedure observation time to be sure there was no bleeding. It went smoothly, proving to be more tedious and annoying than painful, but we'll need to wait 7-10 days for results. It was well into the evening before the Siegels left Boston. After two long and stress-filled days, Susan is, understandably, not at the top of her game. A few days rest and recuperation should see her feeling reasonably decent again.

I'll post again when there's some definitive news from the biopsy, and after that, to provide the outcomes from her next Dana Farber checkup, scheduled for March 8th.

Thursday, January 7, 2010

Yesterday Susan had about a gallon of fluid, over 9 pounds, removed from her abdominal area (it was nearly 4 liters, in medical speak, but we're Americans, so we don't do metric). It's a quick way to lose weight but don't try it at home. This has alleviated the extreme discomfort of the ascites, although it has introduced a new, but temporary discomfort of its own as the various organs and innards resettle into their reconfigured space. That will pass, presumably leaving Susan feeling much more comfortable than she has for a while now.

The MRI revealed a partial thrombosis (i.e., clot) in the portal vein into the liver, as well as scarring on the liver. The doctors don't know at this point what caused the scarring. More information needs to be gathered, which will be done via

a liver biopsy,

an analysis of the withdrawn ascites fluid,

and a "chem 20" blood test (a comprehensive metabolic panel of 20 chemical tests performed on serum (the portion of blood without cells).

The usual treatment for the partial thrombosis can either be blood thinners or simply the passage of time to let the body deal with the matter on its own. Susan's team has decided to hold off (by default, adopting the "time" approach for now), pending the information still to be gathered

Susan's overall blood results from Monday were in or close to normal range, including bilirubin for liver function, so there's no medical emergency involved. The ascites fluid analysis is already in the works. Susan will probably have the chem 20 done at NYU and possibly the liver biopsy as well, but her Boston team is OK with her waiting on these until her next DFCI visit in early February (don't know the exact date yet). By the way, the skin problems reported on previously in this blog are also most likely attributable to the liver problem, rather than GVHD as once speculated, so we'll hope for improvement on that score as well once the liver issue has been successfully addressed.

When I get a firm date for the next DFCI visit and/or other significant information, I'll post it, but as I can't anticipate when that will be, you might want to check on the blog periodically. Of course, the next full report will be after the February visit.

Tuesday, January 5, 2010

Top Line: Blood Tests results were decent; no current evidence of GVHD; liver issue being worked through

Today Susan met with Dr. Cohen, a "really fabulous" hepatologist according to Bob. The results of the late afternoon MRI were not available but, pending those, Dr. Cohen said the problem is likely either a clot in the portal vein or VOD (remember veno occlusive disease?). Whatever the cause of the liver problem, the liver issue is the cause of the ascites. However, he thinks that her test results overall are pretty good.

So, pending the determination of root cause and corresponding treatment, Dr. Cohen wants to eliminate the ascites, which will be done simply by withdrawing all the fluid via a syringe. It could take a while (remember how many pounds of excess fluid Susan carried the last time ascites was an issue), but the procedure is straightforward and not a cause for concern. It is scheduled for 11:00 AM tomorrow, so it's another night in Boston. Once the underlying liver issue is dealt with, the ascites should not return.

I'll blog again tomorrow with what I expect will be the final update for the current DFCI visit.

Monday, January 4, 2010

Susan and Bob were at Dana Farber today as scheduled. Her liver took front an center. As you may remember, the last blog entry indicated that some level of ascites had returned, and you may also remember that the ascites is a result of liver issues.

The ascites has worsened over the past month so Dr. Soiffer had an ultrasound done. The results were inconclusive and he therefore wants Susan to see a liver specialist and have an MRI, which is scheduled for late tomorrow afternoon (the first available slot). The Siegels are staying in Boston tonight and we should know more by tomorrow evening. I'll blog again as soon as I have news.

Susan's disease

Chronic Lymphocytic Leukemia (CLL) is a slowly progressive form of lymphocytic leukemia, characterized by an increased number of B lymphocytes, one of 5 types of white cells in the marrow and blood (hence the concern when white cell counts are elevated). The role of B lymphocytes is to produce antibodies to help combat infectious agents such as bacteria and viruses. With CLL, B lymphocytes multiply unchecked and, in the absence of threats to combat, will attack healthy cells and tissue. It is common to experience enlargement of the lymph nodes and spleen as the disease progresses. CLL is the most prevalent form of leukemia and occurs predominantly after age 55, although Susan, ever the precocious one, was 50 when she was diagnosed.

Susan's diagnosis came in December, 1999, when routine bloodwork revealed an abnormally high white count.After evaluating the several world class cancer centers in the US, she settled on Boston's Dana Farber Cancer Institute (DFCI), affiliated with Harvard Medical School.

Susan was fortunate to be introduced by mutual friends to Dr. Lee Nadler, a Senior Vice President at DFCI and Professor of Medicine at Harvard Medical School. Dr. Nadler, who does not normally take on a patient load, agreed to become Susan's primary oncologist and has, over the years, arranged for her to see the appropriate specialists as needed, whether at DFCI or other institutions.

Susan underwent a regimen of chemotherapy from May through September of 2001 which provided her a strong remission lasting until the Spring of 2005, when elevated bloodcounts indicated that additional treatment was required. Tests revealed that the locus of the disease at that point was in her spleen, and she underwent a successful splenectomy operation in August 2005, enabling her to reestablish her remission.

Of course, there's a wrinkle. Over the past couple of years blood sample analyses have shown the presence of both Hodgkins Lymphoma and non-Hodgkins Lymphoma cells, suggesting that the disease has been morphing, muddying the waters as far as a clearcut diagnosis is concerned. There is no label for what Susan has (we are calling it a variant of CLL, for simplicity's sake). However, it doesn't really matter. The course of treatment at this point is the same: a stem cell transplant.

Although Susan has been fortunate enough to be essentially asymptomatic all these years, her white cell counts have gone up as high as 320,000, only to be brought back down to a reasonable level through chemotherapy. To put that number in perspective, a healthy individual's count usually ranges between 4,000-10,000. The fact that she doesn't look sick and generally feels fine does not mean that she doesn't have a serious and progressive illness.

From the beginning, Dr. Nadler had indicated that at some point the disease would advance to where the best course of action would be a transplant procedure, which is dangerous but potentially curative. That point is now.

Susan's Transplant Procedure

RATIONALE for the TRANSPLANT

While the right protocol of chemotherapy can produce a remission in patients with blood cancers, these remissions are almost always temporary, not curative. The number of remissions a particular patient's disease may enable him or her to realize is very limited - sometimes only one, sometimes several - but in general any successive remission(s) is less robust and of shorter duration. The time comes when the best approach is to go for a cure, which is what a successful blood and marrow stem cell transplant can offer. The transplant entails significant risks (as outlined below), but Susan is at the point where the benefits of another remission (if successfully achieved) might be fleeting and leave her less able to tolerate the transplant at some future date.

BLOOD and MARROW STEM CELL TRANSPLANTS: the SHORT COURSE

Blood, despite its homogenous appearance as a red fluid, is actually a very complex medium that contains many different factors, all of which are generated by a person's bone marrow. In a blood cancer like Chronic Lymphocytic Leukemia (CLL) these components of the blood become unbalanced. A person with CLL produces an ever increasing quantity of white cells in the blood over time, particularly B lymphocytes.

White blood cells , including lymphoctyes (B lymphocytes, T lymphocytes, et al.) act as the body's defense against infection and other foreign bodies, and so they naturally increase to combat such threats, but when the threat has been eliminated, the white cell count returns to its normal level. To oversimplify, elevated levels of white cells, pumped out by malfunctioning bone marrow, with no disease or infection to neutralize, begin attacking healthy tissues and systems. This is the case when a person has CLL.

If the bone marrow which produces the white cells can be restored to its proper functioning, then the white cell count will return to normal. A blood and marrow stem cell transplant can achieve this by largely destroying the sick person's marrow through chemotherapy and/or radiation and then replacing it with healthy marrow. To be more accurate, it is not the physical marrow which is destroyed and replaced but rather stem cells within the marrow responsible for the production of red cells, platelets, and the various kinds of white cells, including the lymphocytes. Therefore, the procedure is most accurately called "Blood and Marrow Stem Cell Transplantation" or Stem Cell Transplantation, although it is often referred to loosely as a Bone Marrow Transplant since the diseased marrow is being regenerated via the healthy stem cells of the donor.

There are two basic kinds of Transplants: autologous and allogeneic. In the autologous version, the patient's own stem cells are harvested during a period of strong remission when healthy stem cells predominate. The blood or marrow with the healthy stem cells is then frozen for later use when the disease has returned in force. In the allogeneic version, the healthy stem cells are provided by a donor whose tissue type closely matches that of the recipient. Susan will be having an allogeneic transplant.

In fact, there are two types of allogeneic transplants - the full or standard transplant (myeloablative transplant) and the so-called "mini-transplant" or "reduced intensity transplant" (non-myeloablative transplant). The standard transplant involves very high doses of chemotherapy and/or radiation in the conditioning phase (the week prior to the transplant), which is very hard on the recipient's system. The mini-transplant involves less intensive conditioning and is better tolerated by older patients (roughly 55 years old and up; Susan, like Jack Benny, is 39 . . . for the 22nd time). The standard transplant works by destroying the diseased bone marrow nearly completely, but with very significant stress on the recipient's system. The mini-transplant works by relying on the donor's immune cells, whose efficacy is bolstered by a drug protocol, to destroy the remaining cancer cells. Susan's medical team has determined that the mini-transplant is the most appropriate procedure for her.

The week or so prior to the actual transplant is referred to as the "conditioning phase" and is used to destroy as many of the cancer cells as possible as well as to suppress the patient's immune system to minimize rejection of the donor's cells. This is done via chemotherapy and/or full body irradiation, and can be very taxing. The actual transplant procedure is similar to a blood transfusion but takes several hours to accomplish.

In the immediate post-transplant period, the patient's immune system is severely compromised and he/she needs to be kept in a protected environment (first the hospital and then a kind of half-way house) and closely monitored. Within a month or so of the procedure, normal blood function is re-established to a degree sufficient to allow the patient to return home, but the environment needs to be very carefully managed to minimize infectious agents. Chronic Graft Versus Host Disease (GVHD - see below for "Risks") can be a significant risk for a year or more after the procedure.

RISKS

The conditioning procedure for an allogeneic stem cell transplant may produce a variety of unpleasant side effects, from mild to quite severe, but these are generally manageable and not considered life threatening. Because it involves a less rigorous conditioning, the "mini" version of the allogeneic transplant (the kind Susan is having) generally causes fewer and less difficult complications. However, there are still three major risks to consider with an allogeneic transplant :

1. Disease or infection.The conditioning process essentially destroys the patient's immune system, leaving him or her virtually defenseless against disease or infection in the pre- and immediate post-transplant period. Therefore, it is extremely important that the patient's environment be carefully controlled to minimize contact with infectious agents. Measures include filtering the air in the room, requiring visitors to wear masks, ensuring frequent and thorough handwashing, eliminating raw foods (e.g., fruits and salads) from the diet, not allowing flowers or plants (which well-wishers might send) into the environment, and so forth. The medical team will also provide the patient with antibiotics and antimicrobial drugs as a preventive measure. It generally takes a month or so for the donor's stem cells to promote formation of enough blood and immune cells in the recipient for him or her to leave the protected environment.

2. Graft Versus Host Disease (GVHD)The allogeneic transplant works largely through an effect called "graft versus tumor" (GVT), where the donor's lymphocytes identify the patient's cancer cells and destroy them. However, the donor's lymphocytes may also attack the patient's normal cells, which they might recognize as a foreign body and so a threat to be neutralized. When this happens, it is called Graft Versus Host Disease (GVHD), a complication that carries a definite mortality risk. The careful tissue matching that is done to ensure donor and recipient compatibility reduces the likelihood and potential severity of GVHD, but it cannot eliminate the risk.

The more complete the tissue matching (it is never 100%, except with identical twins), the healthier the recipient is going in, and the younger the recipient, the milder the case of GVHD is likely to be. Nevertheless, the majority of recipients will experience some degree of GVHD, despite the prophylactic drugs that the medical team administers.

GVHD occurs in two forms -- "acute" and/or "chronic." The terminology has nothing to do with the degree of severity, but rather refers to the timing. Acute GVHD occurs within the first three months, but its likelihood of occurrence diminishes somewhat with time. Chronic GVHD is what develops at some point after the period for the acute phase has passed. The risk for chronic continues for a year or more after the transplant procedure. A person who has developed and been cured of acute GVHD is still at risk for chronic, as is someone who has been symptom free even a year out.

3. Host Versus Graft Effect Since the patient's immune system is not entirely destroyed by the conditioning regimen, it's possible for the remaining cells to reject the transplanted donor stem cells, in which case the transplant is unable to gain traction and do its work. Again, careful tissue matching between donor and recipient will reduce the likelihood of this effect to any worrisome degree, but some level of risk remains.

Blood and Marrow Stem Cell Transplantation - a pamphlet

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